We report a 62-year-old male with MFH of the left ureter, in which a left nephroureterectomy with ... Key words: malignant fibrous histiocytoma, ureteral cancer.
Primary MFH of the Ureter
Case Report
Primary Malignant Fibrous Histiocytoma of the Ureter Ching-Yu Huang1, Victor C. Lin1, Chiang-Ting Wang2, Yu-Ping Tung2, Shang-Tao Chien3 1
Division of Urology, Department of Surgery, E-Da Hospital, Department of Nursing, I-Shou University, Kaohsiung, Taiwan, R.O.C. 2 Division of Urology, Department of Surgery, 3Department of Pathology, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan, R.O.C.
Malignant fibrous histiocytomas (MFHs) are the most common soft-tissue sarcoma of adulthood; they have a male preponderance, and often involve the extremities and retroperitoneum, but a primary MFH of the urinary tract is extremely rare. We report a 62-year-old male with MFH of the left ureter, in which a left nephroureterectomy with bladder cuff resection was performed. The diagnosis was established on the basis of the pathologic microscopic findings and immunohistochemistry. Most studies recommend adjuvant chemotherapy or radiotherapy for these tumors, but the role of these treatments needs further investigation. (JTUA 18:232-4, 2007) Key words: malignant fibrous histiocytoma, ureteral cancer.
INTRODUCTION
CASE REPORT
A malignant fibrous histiocytoma (MFH) was first identified by Ozzello et al.1 and O'Brien and Stout in the 1960s,2 and has been widely regarded as the most common soft-tissue sarcoma of late adult life. MFHs are primitive mesenchymal tumors showing partial fibroblastic and histiocytic differentiation.3,4 Males are more commonly affected than females, with a ratio of 2 to 1. The tumor typically occurs in the lower extremities (49%) (particularly the thigh), upper extremities (19%), or retroperitoneum and abdomen (16%), and arises in the deep fascia (19%) or skeletal muscle (59%).3,4 MFH arising from the genitourinary system is extremely rare, and fewer than 50 cases of renal MFH have been reported in the literature to date. MFH of the bladder or prostate is even rarer, with fewer than 5 cases documented so far. As far as we could determine, primary MFH of the ureter has never been reported before. We present the clinicopathological features of a case of primary MFH of the ureter.
A 62-year-old male presented with painless gross hematuria for 3 weeks. No trauma history was determined before the onset of symptoms. The patient had a history of end-stage renal disease with regular hemodialysis for 4 years, but he denied having any other systemic disease. On a physical examination, there was no tenderness in either the costovertebral angle or palpable flank mass. Laboratory analysis revealed anemia (hemoglobin of 9.4 g/dl) and abnormal renal function, and urinalysis showed microscopic hematuria (32~35 red blood cells per high-power field) without pyuria. The X-ray of kidney, ureter, and bladder was unremarkable. Urine cytology was negative for malignant cells. Left hydronephrosis was found on sonography. Retrograde pyelography demonstrated a filling defect in the left middle third of the ureter (Fig. 1). Ureteroscopy disclosed a widely based soft-tissue lesion with sloughing mucosa at the site of the filling defect. The grasp biopsy presented atypical cells, and malignancy was highly suspected. A computed tomographic (CT) scan revealed a soft-tissue mass in the left middle third of the ureter with moderate hydroureteronephrosis. A left nephroureterectomy with bladder cuff resection was performed under the impression of urothelial carcinoma of the ureter. The ureter was easily separated from the surrounding tissue. The renal pelvis remained intact. The main part of the tumor was elastic in
Address reprint requests and correspondence to: Yu-Ping Tung, MD Department of Surgery, Kaohsiung Armed Forces General Hospital, 2 Chung-Jane First Road, Kaohsiung, Taiwan 802, R.O.C. Tel: 886-7-7492792 Victor C. Lin, MD Department of Surgery, E-Da Hospital, 1 E-Da Road, Jiau-Shu Village, Yan-Chau Township, Kaohsiung County, Taiwan 824, R.O.C. Tel: 886-7-6150011 ext. 2976 Fax: 886-7-6150982
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C. Y. Huang, et al
consistency, measured about 2.5 cm in diameter, and was located in the ureteral wall (Fig. 2). We strongly considered the tumor to have arisen primarily from the ureter. Microscopically, the ureteral tumor arose from the muscular layer, but the overlying urothelium was intact (Fig. 3). Sections of the tumor showed a picture of an undifferentiated high-grade pleomorphic sarcoma composed of marked cytological and nuclear pleomorphism with bizarre giant cells. There was a storiform growth pattern on a myxoid background (Fig. 4). Immunohistochemical results showed that the tumor cells were positive for vimentin and CD68, while negative for smooth muscle actin and S-100 protein. Epithelial markers of pan-cytokeratin (AE1/AE3), CK7, CK20, and EMA were also negative. In addition, the kidney revealed chronic pyelonephritis. The aforementioned histopathologic features suggested a diagnosis of MFH, excluding the possibility of sarcomatoid urothelial carcinoma or other tumor.
Fig. 1.
Retrograde pyelography showing a filling defect (arrow) in the left middle ureter.
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Renal pelvis
Ureter
Fig. 2.
Tumor
An elastic tumor measuring about 2.5 cm in diameter found outside the ureteral lumen. The renal pelvis was intact.
A
B
Fig. 3.
Ureteral tumor arising from the muscular layer, but the overlying urothelium is still intact. A, adventitia side; B, urothelial side (H&E, 40×).
Fig. 4.
Pleomorphic ovoid to spindle-shaped and bizarre giant cells with storiform growth pattern by histology (H&E, 200×).
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Primary MFH of the Ureter
The postoperative course was uneventful. Adjuvant radiotherapy was suggested, but the patient refused. No local recurrence or metastasis was observed during 18 months after the operation.
DISCUSSION Upper urinary tract epithelial tumors involving the ureter are relatively uncommon, occurring with 1/4 the incidence of renal pelvis tumors. Most ureteral cancers are urothelial carcinomas (97%); inverted papillomas, squamous cell carcinomas, and adenocarcinomas rarely occur in the upper urinary tract. Nonurothelial tumors of the upper urinary tract, including fibroepithelial polyps, leiomyomas, angiomas, and leiomyosarcomas, are quite rare, but primary MFH of the urinary tract is extremely rare. In this case, the only presenting symptom was painless gross hematuria. Preoperative imaging studies cannot distinguish MFH from urothelial carcinoma. A definitive diagnosis of MFH depends on pathologic ultrastructural and immunohistochemical studies. A diagnosis of MFH is essentially a diagnosis of exclusion, when dealing with a malignant sarcoma with marked cellular pleomorphism.5 MFH is microscopically characterized by spindle-shaped (fibroblast-like) and round (histiocyte-like) cells arranged in a storiform pattern, accompanied by pleomorphic giant cells and inflammatory cells.3 MFH must be separated from sarcomatoid urothelial carcinoma. Sarcomatoid urothelial carcinoma may be associated with a malignant epithelial component and stains positively for cytokeratin. In contrast, MFH is negative for cytokeratin, and can stain positively for vimentin and CD68. In our case, the urothelium was benign, and the tumor was situated in the muscular layer. The epithelium markers including pan-cytokeratin (AE1/AE3), CK7, CK20, and EMA all showed negative staining. Smooth muscle actin and S100 protein also gave negative results. These findings excluded a sarcomatoid urothelial carcinoma, and MFH was diagnosed. MFH manifests a broad range of histopathological appearances and is classified into several subtypes, known as pleomorphic (70%), giant-cell (10%), and inflammatory types. In addition, the angiomatoid and myxoid types are now considered to be completely separate tumors from other forms of MFH.6 MFH is a fully malignant sarcoma with a 2-year survival of 60%, a recurrence rate of 44%, and a metastatic rate of 42%.3 The causes of local recurrence are an insufficient surgical margin, skip metastasis, regional lymph node metastasis, and tumor emboli.7 A number
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of tumor-associated factors, such as tumor size, tumor depth, histological grade, tumor necrosis, vascular invasion, and mitotic rate, have been identified as prognostic factors for metastasis. The predictive value of the prognostic factors examined was limited to the first 2 years of follow-up.8 MFH of the ureter must be considered a high-grade malignancy. Radical surgery may be the only valid therapy because of the aggressive nature of the disease. To improve the prognosis of MFH, surgical prevention of local recurrence is essential. An adequately wide margin of resection for primary tumors and a curative procedure for recurrent tumors and tumors with an infiltrative pattern should be followed.7 Postoperative radiotherapy can improve local control, but the benefits in increased overall survival rates and distant metastasisfree rates should be further investigated.9 Doxorubicinbased adjuvant chemotherapy appears to significantly improve time to local and distant recurrence and overall recurrence-free survival in adults with a localized resectable soft-tissue sarcoma.10 Several randomized trials are currently underway.
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